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Fetal akinesia deformation sequence

ORPHA:994· ICD-10 Q87.8

Definition

The fetal akinesia/hypokinesia sequence (or Pena-Shokeir syndrome type I) is characterized by multiple joint contractures, facial anomalies and pulmonary hypoplasia. Whatever the cause, the common feature of this sequence is decreased foetal activity.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Antenatal, Neonatal